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Case Report
Cardiology
Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(Suppl 1):S84-S87.   Published online November 30, 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit....

Original Article
Cardiology
Infantile Marfan syndrome in a Korean tertiary referral center
Yeon Jeong Seo, Ko-Eun Lee, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(2):59-64.   Published online February 29, 2016
Purpose

Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea.

Methods

Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated.

Results

Their median age at the time of diagnosis was 2.5 months (range,...

Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Jung Min Go, Jin Su Moon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2015;58(10):392-397.   Published online October 21, 2015
Purpose

Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis.

Methods

We conducted a retrospective study of 41 patients with Alagille syndrome...

The strong association of left-side heart anomalies with Kabuki syndrome
Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
Clin Exp Pediatr. 2015;58(7):256-262.   Published online July 22, 2015
Purpose

Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center.

Methods

A retrospective analysis was conducted for a total of 13 patients with Kabuki...

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea
Bo Kyung Jin, Ji Seok Bang, Eun Young Choi, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Woong Han Kim
Clin Exp Pediatr. 2013;56(3):125-129.   Published online March 18, 2013
Purpose

The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease.

Methods

This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011.

Results

Fifteen patients underwent...

Case Report
Aortic valve replacement surgery for a case of infantile Takayasu arteritis
Hye Won Kwon, Yoon Jung Suh, Ji Seok Bang, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Woong Han Kim, Chung Il Noh
Clin Exp Pediatr. 2012;55(7):254-258.   Published online July 17, 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed...

A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction
Byeong Sam Choi, Bo Sang Kwon, Gi Beom Kim, Yoon Kyung Jeon, Jung-Eun Cheon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 2009;52(9):1029-1034.   Published online September 15, 2009
Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was...
Pheochromocytoma associated with cyanotic congenital heart disease
Seung Joon Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, Eun Jung Bae, Jung Il Noh
Clin Exp Pediatr. 2008;51(1):93-97.   Published online January 15, 2008
Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition,...
Original Article
Follow-up Study of Children with Anthracycline Cardiotoxicity
Hyok Joo Kwon, Young Hwan Song, Soo Jung Kang, Hyoung Jin Kang, Hyoung Soo Choi, Eun Jung Bae, Hee Young Shin, Chung Il Noh, Yong Soo Yun, Hyo Seop Ahn
Clin Exp Pediatr. 2003;46(3):242-249.   Published online March 15, 2003
Purpose : We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. Methods : A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two...
Study for Balloon Aortic Valvuloplasty in Congenital Aortic Stenosis
In Seung Park, Do Jun Cho, Mi Young Han, Jae Young Lee, Soo Jin Kim, Mee-Hye Oh, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2001;44(2):167-176.   Published online February 15, 2001
Purpose : The aims of this study are to investigate the results of balloon aortic valvuloplasty (BAV) in congenital aortic stenosis(CAS) and, especially, to compare the results between BAV performed before two months of age(Group A) and BAV after two month of age(Group B). Methods : From January 1993 to June 2000, 14 patients who were diagnosed as CAS were treated...
Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography
Jung Yun Choi, Jeong Jin Yu, Soo Jung Kang, Jae Seong Son, Young Mee Seo, Jin Young Song, Ho Sung Kim, Eun Jung Bae, Chung Il Noh, Yong Soo Yun
Clin Exp Pediatr. 2001;44(2):154-160.   Published online February 15, 2001
Purpose : Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. Methods : This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations...
Study of Congenital Mitral Stenosis Cases Requiring Surgical Correction in the First 2-Years of Life
In Seung Park, Young Seok Lee, Mi Young Han, Jae Young Lee, Soo Jin Kim, Do Jun Cho, Mee-Hye Oh, Woong-Han Kim, Young-Tak Lee, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(11):1458-1464.   Published online November 15, 2000
Purpose : Congenital mitral stenosis(CMS) is a rare anomaly accounting for 0.4-0.5% of total heart disease. CMS which cases needed surgical correction in infancy are even rare. In this study, we analyzed 11 CMS patients of less than 2 year of age who needed surgical corrections, in order to find out their diagnoses, the characteristics, the results of operation and...
Follow up of Patients with Total Anomalous Pulmonary Venous Return in Right Atrial Isomerism
Soo Jin Kim, Jae Young Lee, Mi Young Han, Do Jun Jo, In Seung Park, Mee-Hye Oh, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(11):1451-1457.   Published online November 15, 2000
Purpose : Total anomalous venous return(TAPVR) is associated in more than 60% of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism. Methods : Between February 1991 and...
Early Results of Stent Implantation in Branch Pulmonary Artery Stenosis after Tetralogy of Fallot Repair
Sung Kyu Lee, Sang Hun Lee, Jae Young Lee, Su Jin Kim, Mi Young Han, Do Jun Cho, In Seung Park, Eun Jung Bae, Sung Ho Kim
Clin Exp Pediatr. 2000;43(10):1343-1349.   Published online October 15, 2000
Purpose : Pulmonary artery stenosis is mainly complicated by aortopulmonary shunt or total correction of tetralogy of Fallot(TOF). Results of surgical angioplasty are poor and the success rate of balloon angioplasty is 53-72%. Endovascular stents have been applied to these lesions. The purpose of this study is to evaluate the early results of stent implantation in postoperative pulmonary artery stenosis...
Case Report
A Case of Transcatheter Occlusion of Aortopulmonary Window(APW) after APW Banding
Young Seok Lee, In Seung Park, Jae Young Lee, Soo Jin Kim, Mi Young Han, Do Jun Cho, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(9):1290-1293.   Published online September 15, 2000
Aortopulmonary window is an uncommon cardiac anomaly accounting for approximately 0.2-0.6% of all congenital cardiac anomalies in which there is a connection between the ascending aorta and pulmonary trunk. Since the first report of successful surgical repair, many investigators have advocated surgical closure of all types of aortopulmonary windows using different technique. The majority of aortopulmonary windows require surgical therapy...
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